Abstracts

Rationale: Sturge-Weber Syndrome (SWS) is a rare neurocutaneous disorder caused by focal vascular dysplasia. It is associated with facial angiomas, ocular abnormalities, epilepsy, intellectual disability, and leptomeningeal angiomas. There is a paucity of literature regarding SWS with epilepsy and pregnancy. The effects of pregnancy on this syndrome therefore remain largely undefined. Consensus on management is needed. This is a case report of a woman with SWS, refractory epilepsy, and successful pregnancy and delivery.

Methods: Data were collected from medical records at the University of Cincinnati, including obstetrics, anesthesia, neurology, and neuroimaging. MG provided written and informed consent for this report.

Results: MG is a 41-year-old G1P1001 left-handed female. She has predominantly focal seizures with abnormal sensation of the right arm or arm jerk since she was one year old. Her seizures were difficult to control through age 14. She is high functioning, and her examination is remarkable for a left VI port wine stain with asymmetry of her left forehead and eye. Her brain MRI shows two large venous anomalies in the left frontal and parietal lobes, thick leptomeningeal enhancement in the left frontal lobe with mild atrophy and prominent medullary veins of the left cerebral hemisphere. She was treated with brivaracetam, lamotrigine, and clobazam throughout her pregnancy with stable seizure control. Pregnancy was significant for a fetal weight in the 95^th percentile for selected gestational age, dilated umbilical vein, and mild polyhydramnios. She underwent an uncomplicated, scheduled low transverse cesarean section at 39 weeks, giving birth to a healthy boy. Anesthetic for her cesarean section was achieved via single shot spinal anesthesia which included hyperbaric bupivacaine, duramorph, fentanyl, and epinephrine intrathecally. There were no neurologic complications and she remained normotensive without medications. Her lamotrigine dose was adjusted postpartum without complications.

Conclusions: This is a case of uncomplicated delivery via cesarean section in a patient with refractory epilepsy and SWS. The case highlights the importance of getting prepartum neuroimaging with MRI brain with known SWS, as this will help to risk stratify and prevent complications during gestation and delivery. A more controlled method such as cesarean section may be selected for a patient with large angiomas or a history of intracranial hemorrhage. If general anesthesia is used, care should be taken to avoid agents that can increase intracranial pressure and thereby increase risk of intracranial hemorrhage. In a reported case of intraventricular hemorrhage after cesarean section, the patient had a trigeminal port wine facial nevus but had not yet been diagnosed with SWS (Dolkart LA, Bhat M, 1995). The only reported case of uncomplicated vaginal delivery in SWS was in a patient who had the angiomatosis surgically removed in childhood (Aziz AS, Hui D, Chinnappa V, Aviv RI, Swartz RH, 2013). There is not enough information available to determine a preferred method at this time, but this case supports that a successful pregnancy and delivery is attainable with SWS and refractory epilepsy.

Funding: Please list any funding that was received in support of this abstract.: none.